03.07 Scleroderma

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Overview

  1. Scleroderma
    1. Autoimmune disease
    2. Too much collagen in tissues

Nursing Points

General

  1. Autoimmune disease
    1. Stressor-> activation of T cells
    2. Overproduction of collagen in tissues
    3. Hardening and tightening of tissues
      1. Skin
      2. Blood vessels
      3. Organs
        1. Lung fibrosis
        2. GI tract
        3. Kidney (affected blood flow) ->sclerodermic renal crisis
      4. Fingers/toes (Raynaud’s)
      5. Vaginal opening – very painful
  2. Limited
    1. Less widespread
    2. Slow developing
  3. Diffuse
    1. Rapid skin thickening
    2. More widespread

Assessment

  1. Check pulses and skin (restricted bloodflow)
    1. Morphea – waxy patches on skin
    2. Linear scleroderma- streak of hard waxy skin
  2. Respiratory status
    1. Respirations
    2. Pulse oximetry
  3. Kidney function
    1. BUN and Creatinine (damaged kidneys aren’t filtering out creatinine and BUN)
    2. Protein in urine (damaged kidneys aren’t preventing protein release)
  4. Heart function
    1. Rate and rhythm
    2. Hypertension (tightened vessels)
  5. CREST Syndrome
    1. Calcinosis (calicum deposits in connective tissue)
    2. Raynaud’s (cold white/blue hands and feet)
    3. Esophageal dysmotility (difficulty swallowing)
    4. Sclerodactyly (tight thick skin on fingers)
    5. Telangiectasia (very visible swollen small blood vessels)

Therapeutic Management

  1. Medications
    1. Steroids ->decrease inflammation
    2. Blood pressure meds ->dilate vessels
    3. Immune system suppression meds (methotrexate) ->stop/slow T cell attack
    4. Pill to decrease acid in stomach (tightened esophagus)
    5. Pain relief
  2. Surgery
    1. Amputation
    2. Lung transplant

Nursing Concepts

  1. Immunity -> autoimmune disorder involving T cells
  2. Tissue/Skin Integrity ->excess collagen in tissues
  3. Gas exchange -> pulmonary fibrosis = lung thickening and tightening

Patient Education

  1. Stay active
  2. Skin care
  3. Avoid stressors
  4. Protect from cold

Reference Links

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Video Transcript

Hey guys! Welcome to the lesson on scleroderma where we will explore what this disease is and how it affects the body.

Scleroderma is an autoimmune disease that is thought to be triggered by some sort of stressor and results in T cell attack on normal cells. Collagen is overproduced in all tissues causing hardening and tightening of the tissues of the body. The skin is the most obviously affected as you will notice the shiny skin patches. The blood vessels are thickened and tightened causing high blood pressure. Lungs become fibrosed where the lung tissues are like scar tissue. The GI tract is affected, especially the esophagus causing reflux of acid. The kidneys end up with decreased blood flow due to the affected vessels which will result in sclerodermic renal crisis and eventually renal failure. Let’s explore two different types of scleroderma. 

So if the patient has limited scleroderma, it develops slowly and is less widespread. Diffuse scleroderma is much more widespread and comes about quickly. Now let’s talk about the patient assessment. 

We will assess this patient’s skin for morphea or linear scleroderma. Morphea are waxy patches on the skin, and linear scleroderma shows as streaks. We want to assess their respiratory status, especially if they have that pulmonary fibrosis. Check their respirations, is it hard for them to breath? Do they need oxygen? Maybe keep a continuous pulse oximeter on to monitor their oxygen levels. The doctor may order a lab draw for BUN and creatinine to assess kidney status. Elevated BUN and creatinine levels signal that the kidneys are damaged and not filtering the muscle and protein breakdown byproducts out of the body. Listen to the heart rate and rhythm. Is there blood pressure elevated? These are all things to look for. Let’s now explore a mnemonic to help remember the effects of scleroderma.

CREST syndrome is a mnemonic often used to remember the effects of scleroderma. Calcinosis is the deposits of calcium in the connective tissue which can be pretty painful. Raynaud’s is where the hands and feet are often cold from lack of circulation. Esophageal dysmotility is where the patient has a hard time swallowing from the tightened esophagus. Sclerodactyly is the tight thick skin on the fingers. Lastly, telangiectasia are those visible swollen small blood vessels like people sometimes get in their face like spider veins. Now how will we manage scleroderma?

We may administer medications that the doctor orders like steroids to help decrease inflammation from the autoimmune attacks. Vasodilator blood pressure drugs will help open up those stiff hard vessels. Immune system suppressors like methotrexate might be used to slow the T cell attack on the healthy cells. Acid reducers like omeprazole may be used for any acid reflux the patient has due to that tight esophagus. Pain relief may be needed, especially for the patient that gets the collagen buildup in the vaginal opening. I have had a few patient’s in the past with this, and it is very painful to the point where the slightest touch hurts. Surgery may be necessary if the disease progresses to be severe, like amputation or an organ transplant. Now let’s discuss patient education. 

So this patient should stay active, even through the pain they may have to try and battle any stressors initiating this autoimmune response in the body. They should avoid stressors like smoking, alcohol, and psychological stress that may worsen the condition. The doctor may recommend special skin care like steroid cream to help decrease inflammation. Protection of cold will help to decrease the effects of the Raynaud’s on the fingers and toes where there is decreased blood flow. 

Our priority nursing concepts for the patient with scleroderma are immunity, tissue/skin integrity, and gas exchange.

Alright, let’s review the key points. Scleroderma is an autoimmune disease involving the T cell attack on healthy cells causing the overproduction of collagen in all body tissues. It may be limited which is slow to come on and not as widespread, or diffuse which comes on quickly and spreads all over.

The patient may have morphea which are waxy patches from the collagen buildup, or linear spots. We should assess the patient’s lungs for shortness of breath and oxygen levels because remember that build up of collagen in the lungs causes pulmonary fibrosis which is not stretchy so it’s hard to expand the lungs. Assess the heart for irregular rates or rhythms and the blood pressure will likely be high from the tight thick vessels in the body. The patient’s kidney function may be evaluated by the doctor ordering labs for BUN and creatinine because the lack of blood flow to the kidneys may result in kidney failure.The CREST syndrome mnemonic stands for results of scleroderma which are calcinosis, Raynaud’s, esophageal dysmotility, sclerodactyly, and telangiectasia. The disease may be managed with stress management, blood pressure meds, autoimmune medications, and steroid creams for the skin.

Well, that’s it on scleroderma! No go out and be your best self today, and as always, happy nursing!

Read more

  • Question 1 of 5

The client with scleroderma in the vagina is admitted to the hospital for pneumonia. Which of the following care plans should the nurse implement?

  • Question 2 of 5

A client is admitted with scleroderma. Which of the following medications would the nurse expect to be ordered for the client?

  • Question 3 of 5

A nurse caring for a client with scleroderma knows that which of the following side effects might be assessed? Select all that apply.

  • Question 4 of 5

A nurse is assessing a client with scleroderma and knows that the client is at high risk for which of the following?

  • Question 5 of 5

A client has severe lung fibrosis and as the nurse completes the admission history, which of the following diseases should the client be asked about?